Degradation of GABA

Stable Identifier
R-HSA-916853
Type
Pathway
Species
Homo sapiens
Compartment
ReviewStatus
5/5
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this pathway in the Pathway Browser
GABA is metabolized in the mitochondrial matrix to succinate by the serial action of two enzymes, 4-aminobutyrate aminotransferase and suucinate semialdehyde dehydrogenase. Failure of the second reaction is associated with a rare human genetic disorder (Malaspina et al. 2016; Pearl et al. 2009).
Literature References
PubMed ID Title Journal Year
19172412 Succinic semialdehyde dehydrogenase deficiency: lessons from mice and men

Pettiford, JM, Forester, K, Wu, Y, Theodore, WH, Knerr, I, Carter Snead O, 3rd, Cortez, MA, Gibson, KM, Pearl, P L, Jakobs, C

J Inherit Metab Dis 2009
27311541 Succinic semialdehyde dehydrogenase deficiency (SSADHD): Pathophysiological complexity and multifactorial trait associations in a rare monogenic disorder of GABA metabolism

Roullet, JB, Gibson, KM, Ainslie, GR, Pearl, P L, Malaspina, P, Vogel, KR

Neurochem. Int. 2016
Participants
Participates
Event Information
Orthologous Events
Authored
Reviewed
Created
Cite Us!